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67 

Techniques for Cleft Lip Repair


David Drake, Serryth Colbert

KEY POINTS
• The objectives of cleft lip repair are to reestablish an addressing the medial shortening and lateral displacement
anatomic and functional balance between the soft tissues while restoring normal anatomy, lip length, and symmetry.
and the skeleton. This is achieved by reestablishing normal • Repair of a bilateral cleft lip is characterized by a huge
insertions of all the nasolabial muscles and correct diversity of treatment protocols that are often associated
anatomical position of the soft tissues recreating the with more long-term issues than unilateral clefts.
appearance of the non cleft lip. • Nasoalveolar molding (NAM) is promising but
• We advocate an advancement/rotation repair of the objective analysis of long-term outcome has not yet been
unilateral cleft lip incorporating an inferior triangular flap reported.

INTRODUCTION uninterrupted movement of mesenchymal cells between the


medial and lateral components of the upper lip by 8 1 2 weeks
Clefts of the lip and/or palate are the most common craniofacial postconception (see Figure 67-1).5
birth anomalies and are among the most common of all birth Cleft lip can be caused by:
anomalies, with prevalence ranging from 1 in 500 to 1 in 2000 • Abnormal growth in the direction of the facial
births, depending on the population.1 Successful management prominences
of patients born with cleft lip and/or palate requires multidis- • Change in position of the nasal placodes6-8
ciplinary, highly specialized team management from birth to • Hypoplasia or abnormal directional growth of mesenchymal
adulthood. An understanding of embryology and anatomy of processes
the cleft deformity is essential in order to optimize surgical • Failure of fusion or breakdown of fusion of mesenchymal
treatment of cleft lip. The challenges and technique of repairing processes6,8,9
unilateral and bilateral cleft lips are outlined. Disruption of matrix metalloproteinase enzyme activity and
its effect on the medial edge epithelium is an example of a pos-
sible common pathway for the range of genetic and environ-
EMBRYOLOGY OF CLEFT LIP mental influences that result in cleft palate.10 The severity of the
The primitive craniofacial complex forms during week 4 post- deformity is related to the amount, timing, and location of the
conception after neural crest tissue migration, early brain vesicle embryonic interruption.11
enlargement, and craniocaudal and lateral trunk folding of the
trilaminar disc. A series of inductive events occur between CLASSIFICATION OF CLEFT LIP AND PALATE
the prosencephalon, mesencephalon, and rhombencephalon.
The neural crest tissue that migrates into the craniofacial Cleft lip and/or palate can be divided into two groups
complex and pharyngeal arch apparatus helps to form the five (Figure 67-2):
facial prominences, the frontonasal and the bilateral maxillary • Primary palate—lip, alveolus, and hard palate anterior to
and mandibular prominences (Figure 67-1).2 It is the differen- incisive foramen (i.e., the premaxilla)
tiation, growth, and eventual fusion of these prominences that • Secondary palate—hard and soft palate posterior to incisive
forms the definitive face. foramen
The upper lip is completed on either side of the globular
prominence (see Figure 67-1) by fusing with the freely project- Primary and Secondary Palate
ing medial nasal prominences of the frontonasal prominence.3 Many different classification systems of varying complexity
Such fusion requires critically timed coordination of growth have been proposed for cleft lip and palate (see Figure 67-2).
between the processes, exact spatial localization, and apoptosis Veau’s 1931 classification, which has four categories, is an
(or further differentiation) of the epithelium that forms the example of a simple system (Figure 67-3)12:
transient nasal bridge or fin between the two processes.4 • Group I: Defects of the soft palate only
The degradation of the underlying nasal fin allows for the • Group II: Defects involving the hard palate and soft palate

948
CHAPTER 67  Techniques for Cleft Lip Repair 949

A B
FIG 67-1  Development of the facial processes. A, Representation of a 30- to 32-day-old human
embryo showing the development of the facial processes. FNP, Frontal nasal process; LNP,
lateral nasal process; MAN, mandibular process; MAX, maxillary process; MNP, medial nasal
process. B, Representation of an 8 12 -week-old embryo showing the fate of the facial process.

Bilateral 
Right + Left

Nose Median

Right Left Primary palate


Lip

Alveolus
Incisive fossa
A B
Hard palate

Secondary palate
Soft palate

Line of cleft

FIG 67-2  Primary and secondary palate.

• Group III: Defects involving the soft palate to the alveolus,


usually involving the lip
• Group IV: Complete bilateral clefts C D
FIG 67-3  Veau classification. A, Group 1 – defects of the soft
Veau’s Classification palate only. B, Group 2 – defects involving the hard and soft
Veau’s classification is incomplete, because it makes no palate. C, Group 3 – defects involving the soft palate to the
provision for isolated cleft lip or alveolus (see Figure 67-3). alveolus, usually involving the lip. D, Group 4 – complete bilat-
Further cleft lip and palate classifications were described eral clefts.
by Fogh-Andersen in 1942, Kernahan and Stark in 1958, and
Pfeifer in 1971.13-16 ting one H from the acronym LAHSHAL to become LAHSAL.
Kriens introduced a simple coding system for paraphrasing The LAHSAL code is based on the striped Y diagrammatic
cleft lip and palate in 1989.17 This system represents the classification (Figure 67-4).
anatomical site, the side, and type of cleft (complete, partial, The relevant parts of the mouth are subdivided into six
microform) in a simple alphabetical code: LAHSHAL. This parts:
classification was modified on the recommendation of the 1. Right lip
Royal College of Surgeons (United Kingdom) in 2005 by omit- 2. Right alveolus
950 CHAPTER 67  Techniques for Cleft Lip Repair

3. Hard palate
4. Soft palate Examples of the LAHSAL Cleft Classification
5. Left alveolus Some examples of the LAHSAL cleft classification are as follows:
6. Left lip • Bilateral complete cleft of lip and palate: A complete bilat-
The code is then written as if looking at the patient. The first eral cleft lip and palate is represented by all six capital
character is for the patient’s right lip and the last for the left lip. letters—LAHSAL.
Complete clefts are recorded in upper case and partial clefts in • Right incomplete cleft lip: A right incomplete cleft lip is
lowercase, and microform clefts are denoted by an asterisk. No represented by the lowercase letter “l” only. Because it is on
cleft is represented with a dot. This classification system is used the right, the “l” is written at the beginning—“l….”
in the United Kingdom because it has the advantage of being • Left complete cleft lip and palate: A left complete cleft lip is
easy to record in medical records and electronically. represented by the capital letters HSAL. Because it is on the
left, the HSAL is written at the end—“…HSAL.”
• Left incomplete cleft lip and alveolus: A left incomplete cleft
lip and alveolus is represented by the lowercase letters “a” and
“l” and they are written at the end—“…al.”
• Incomplete hard palate and complete soft palate cleft: An
incomplete cleft of the hard palate is represented by the
lowercase letter “h,” and the complete cleft of the soft palate
is represented with the capital letter “S”—“…hS….”

UNILATERAL CLEFT LIP ANATOMY


Skin Anatomy
The anatomy of the skin of the unilateral cleft lip is character-
ized by shortening of the skin on the medial side of the cleft lip
with lateral displacement of the skin on the lateral side of the
cleft (Figure 67-5). The skin is retracted and displaced and is
associated with sterile mucosa along the cleft margins on both
sides of the cleft. There is vertical soft tissue deficiency on the
medial aspect of the cleft and a discontinuity of skin, muscle,
and oral mucosa on the upper lip on the cleft side. The junction
between the skin and vermilion forms a pale and raised ridge
to a variable degree in continuity with the white roll.

Muscle Anatomy
The upper lip is formed by a series of three interconnected
muscle rings extending from the infraorbital rim and nose
down to the chin (Figure 67-6, A). These nasolabial
FIG 67-4  LAHSAL cleft classification. muscle rings form part of the fascial envelope in continuity

Lateral Shortening of
displacement of skin of medial
skin on the side with
lateral side vertical soft
tissue
deficiency
Pale raised
ridge
Sterile
mucosa
Sterile
mucosa

White roll

FIG 67-5  Anatomy of skin in unilateral cleft lip.


CHAPTER 67  Techniques for Cleft Lip Repair 951

A B
FIG 67-6  A, Nasolabial muscle ring in the non-cleft lip. B, Nasolabial muscle ring in the unilateral
cleft lip.

Separation of domes of the alar cartilage


at the nasal tip and retroplacement Deflection of the nasal tip
of the cleft alar cartilage dome toward the non-cleft side
Rotation of septum, columella, and
Kinking of the lateral crus on the cleft side nasal spine away from cleft with
forming obtuse angle with medial crus shorter columella on the cleft side

Dislocation of the caudal portion


of the septum to the non-cleft side
Displacement of alar base in all from the nasal spine
three planes

Larger nares on the cleft side Inferior displacement of the


medial crus within the columella

FIG 67-7  Nasal anatomy of the unilateral cleft lip.

with the superficial musculo-aponeurotic system (SMAS) (see of the cleft. These muscle fibers are interspersed with connective
Figure 67-6, A). tissue.
In the unilateral cleft lip and palate, the upper and middle Some general observations from the perspective of cleft lip
muscle rings are incomplete (see Figure 67-6, B). All the muscle repair are apparent:
groups on the cleft margins of the cleft, which normally insert • The upper and lower muscle rings converge toward the ANS
onto the anterior nasal spine (ANS), septum, and anterior and nasal septum.
surface of the premaxilla, become bunched up at the border of • The transverse nasalis muscle intermingles with the levator
the cleft. The abnormal muscle function produces characteristic labii superioris and levator labii superioris alaeque nasi to
nasal and mucocutaneous abnormalities, which have to be form a modiolus that fans out to insert into the nasal sill. It
addressed at the time of primary lip repair. The unilateral cleft influences the shape and position of the ala cartilage and the
lip is characterized by discontinuity, disorientation, and mal height of the nasal sill.
insertion of the orbicularis muscle, causing horizontal and ver- • The almost vertical orientation of the external or superficial
tical lip discrepancy. In a complete cleft lip, the deep fibers of part of the orbicularis oris and its connections with the
the orbicularis oris muscle are interrupted by the cleft. In addi- muscles of the upper ring and with the lower ring through
tion, the superficial component of the orbicularis oris turns the modiolus are significant.
upward, along the margins of the cleft, and it ends beneath the
base of the ala or columella. Nasal Anatomy
Incomplete cleft lip behaves in a similar manner, except The obvious deformity of nasal form in unilateral cleft lip and/
when the cleft is less than two-thirds of the height of the lip. In or palate is due to the incorrect insertion of the transverse
this case, the fibers of the muscle run along the margins of the nasalis muscle and displacement of the septum (Figure 67-7).
cleft, then change direction and run horizontally over the top The muscle in the lateral element is deprived of insertion to the
952 CHAPTER 67  Techniques for Cleft Lip Repair

ANS and pulls the lateral lip element and alar base of the nose UNILATERAL INCOMPLETE CLEFT LIP ANATOMY
laterally. The alar cartilage is flattened and deformed but is not
hypoplastic. The lateral crus of the lower lateral cartilage is Microform cleft lip involves partial or total clefting of the upper
pulled laterally and lengthened at the expense of the medial crus lip musculature (Figure 67-8). Incomplete cleft lip involves skin,
thereby flattening the dome. Its inferior border is also rotated muscle, and mucosa, but it may spare the underlying skeletal
inferiorly forming a web inside the nostril. The anterior nasal structures (Figure 67-9). Complete unilateral cleft lip involves
septum and columella are deviated to the medial side. The skin, muscle, mucosa, and underlying skeletal framework.
anterior part of the greater segment of the maxillofacial complex There are subtle textural differences between the nasal skin
is displaced anteriorly due to lack of molding of the nasolabial and lip skin in an incomplete cleft lip. During the repair of a
muscles. cleft lip, they are separated at their junctions to align nasal skin
The anatomical features of the nose in unilateral complete with nasal skin and lip skin with lip skin.
cleft lip are illustrated in Figure 67-7 and are listed in Box 67-1. Muscle dissections in stillborn babies have shown that the
upper lip muscles in incomplete cleft do not cross the cleft gap
unless the skin bridge is greater than one-third of the height of
BOX 67-1  Anatomical Features of the
the lip.18 Even if orbicularis oris muscle is present in the skin
Nose in Unilateral Cleft Lip bridge, the orientation of the fibers is abnormal and the mus-
Abnormal muscle insertions into nasal spine away from cleft culature is not functionally correct. Therefore repair of the
Defect in primary palate anterior to incisive foramen incomplete cleft lip requires a full muscle dissection on the
Rotation of septum, columella, and nasal spine away from cleft medial and lateral segments to allow for a functional muscle
Separation of domes of the alar cartilage at the nasal tip and kinking of
the lateral crus on the cleft side
repair across the cleft.
Dislocation of lower and upper lateral cartilages on the cleft side
Displacement of alar base in all three planes BILATERAL CLEFT LIP ANATOMY
Inferior displacement of the medial crus within the columella
Dislocation of the caudal portion of the septum to the non-cleft side Skin
from the nasal spine The anatomic skin characteristics of bilateral cleft lips are deter-
Displacement and flattening of the nasal bone on the cleft side
mined by the degree of completeness of the cleft and its sym-
Deflection of the nasal tip toward the non-cleft side
Retroposition of the cleft alar cartilage dome metry. The prolabial skin in bilateral cleft lip varies in size, is
Obtuse angle between the medial and lateral crura of the lower lateral often retracted, and lacks muscle fibers (Figure 67-10). In addi-
cartilage on the cleft side tion, the columella is shortened, and the prolabium appears
Absence of the alar-facial groove on the cleft side and attachment of attached to the top of the nose in some cases. The size and posi-
the ala to the face at an obtuse angle tion of the premaxilla varies but it is rotated upward and pro-
Larger nares on the cleft side
truded. In some cases, it can be excluded from the alveolar arch
Shorter columella on the cleft side, positioning the entire columella at
a slant toward the non-cleft side
with a collapse of the lateral elements. The isolated prolabium
is deprived of muscle and normal vermilion and is lined by

Partial or total cleft


of upper lip musculature

FIG 67-8  Microform cleft lip.


CHAPTER 67  Techniques for Cleft Lip Repair 953

Nasal skin
Absence of functional
muscle in skin bridge
Lip skin

FIG 67-9  Incomplete cleft lip.

Apparent
absence of
columella

Abnormal
Retracted prolabial vermilion
skin lacking continuity of lined by
orbicularis oris muscle fibers sterile
mucosa

Protrusion
and rotation Collapse of
of premaxilla lateral palatal
segments
behind the
premaxilla

FIG 67-10  Skin anatomy of bilateral cleft lip and palate.

sterile mucosa. The prolabium located centrally is devoid of segments, the lip vermillion is turned upward to join the alar
muscle and consists of skin, vermilion, and oral mucosa. base. This is because the levator alaeque nasi, levator labii supe-
There is no definitive line of demarcation between the colu- rioris, transverse nasalis, and intrinsic upper lip muscles orbi-
mella and prolabium. The premaxilla may be situated centrally, cularis marginalis are oriented upward and inserted into the
asymmetrically to the right or left, anteriorly, superiorly, or alar base and maxilla along the pyriform ring (Figures 67-12,
inferiorly, or a combination of thereof (Figure 67-11). 67-13, and 67-14), resulting in an absence of muscle in the
prolabial segment.
Muscle
The nasolabial muscle rings are disrupted in complete bilateral Nose
cleft lips, and their abnormal insertions result in lateral dis- The nasolabial deformity in bilateral cleft lip and palate is
placement of the lateral nasolabial elements. On the lateral largely caused by secondary hypoplasia rather than primary
954 CHAPTER 67  Techniques for Cleft Lip Repair

Protrusion and anterior


rotation of the premaxilla

FIG 67-11  Rotation and protrusion of the premaxilla on lateral view.

A B
FIG 67-12  A, Nasolabial muscle ring in the non-cleft lip. B, Nasolabial muscle ring disruption in
a complete bilateral cleft lip.

mesenchymal deficiency.19 The most important feature of the 67-15). The lower lateral cartilages are pulled laterally, resulting
bilateral cleft is the short columella that is produced by an unop- in a flattened appearance of the nose. The septum and premax-
posed muscular pull on elements of the lateral cleft in the early illa are attached to the vomer as a thin stalk. Alar domes and
stages of fetal development. The bilateral nasal cleft is seldom middle crura are splayed, caudally rotated (bucket handle), and
corrected by functional surgery alone, and the appearance of a subluxed from their normal anatomic position overlying upper
repaired bilateral cleft lip is often dominated by the short, teth- lateral cartilages. The nasal tip appears large, flat, and bifid,
ered appearance of the columella, and a broad nasal tip (Figure because both alae are rotated downward and spread apart.
CHAPTER 67  Techniques for Cleft Lip Repair 955

PHILOSOPHY OF CLEFT LIP REPAIR quadrangular flap (Le Mesurier), or a combination of the
advancement rotation and wavy line techniques (Afroze).20-24
The essential objectives of cleft lip repair are the restoration of The skin of the lip on the medial element is short as a con-
normal function and appearance of the lip and nose. This is sequence of the change in normal muscular actions. Careful
achieved by reestablishing normal insertions of all the nasola- reconstruction of the nasolabial muscles allows the overlying
bial muscles and correct anatomical position of the other soft skin to lengthen to the correct lip height.
tissues, including the mucocutaneous elements. Therefore, an • Muscle: The fundamental goal of surgery is to achieve ana-
anatomic and functional balance between the soft tissues and tomic muscular reconstruction, particularly with respect to
the skeleton is reestablished. anchorage of the complex nasolabial muscles of the cleft side
• Skin: The skin is corrected in both vertical and horizontal to the nasal septum and muscles on the non-cleft side (see
orientation. To restore normal height of both sides of the Figure 67-15). It is necessary to reconstruct the nasolabial
cleft relative to the non-cleft side, it is necessary to restore muscles of the cleft such that the skin margins are not under
the normal length of the skin on the cleft side using various
techniques, such as the rotation advancement design (Delaire
and Millard), undulating wavy flaps (Pfeiffer), straight line
design (Rose-Thompson), lower lip Z-plasties/triangular
flaps (Tennison-Randall, Skoog, Trauner, or Malek), the

Absence of muscle in the Lateral displacement and


prolabial segment insertion of nasiolabial
muscles into the alar base

FIG 67-14  Nasolabial muscle ring disruption in the bilateral


FIG 67-13  Absence of muscle in the prolabial segment. cleft lip.

Broad, flat and


bifid nasil tip

Short tethered
columella

Caudal rotation of
splayed alar domes

“Thin stalk” -like septal


attachment of premaxilla
to vomer

FIG 67-15  Muscle anatomy in unilateral cleft lip.


956 CHAPTER 67  Techniques for Cleft Lip Repair

tension at the moment of skin closure (Figure 67-16). If the • Muscle attachments of the medial side: The most nasal
primary nasolabial muscle reconstruction is good, anatomy, and deep bundles of orbicularis muscle insert into the
function, skeletal growth, and total facial esthetics can be mucoperichondrium and anterior nasal septum. There-
excellent. fore, they pull the caudal part of the septum toward the
• Nose: The nasal septum is deviated in unilateral cleft lip. medial side.
There are two reasons for this: Septal deviation causes the upper lateral cartilage to buckle,
• Position of the ANS: It is positioned toward the non-cleft depressing the radix, and also causes the lower lateral cartilagi-
side and therefore pulls the septum, which is attached to nous framework to shift, thereby collapsing the dome on the
it toward the non-cleft side. lateral side (Figure 67-17).

Aim is to anchor muscles on cleft side to the nasal


Functional reconstruction of muscles with skin margins lying passively
septum and muscles on the non-cleft side

A B
FIG 67-16  A, Functional muscle reconstruction in unilateral cleft lip. B, Muscle in repaired uni-
lateral cleft lip.

Depression of radix due


to buckling of upper
lateral cartilage
Septal deviation to
the medial side

Collapse of dome lower


lateral cartilage on
lateral side

FIG 67-17  Deviation of nasal septum to non-cleft side.


CHAPTER 67  Techniques for Cleft Lip Repair 957

Septoplasty at the time of lip repair produces a straighter continuity with the floor of nose. There is no evidence to
nasal septum positioned in the facial midline and restores the support the hypothesis that vomer flaps restrict maxillary
patency and natural width of the nostril (Figure 67-18). To growth.25
achieve the proper midline position and attitude of the nasal
septum, the surgeon must perform a wide subperichondrial Alveolar Cleft Segments
dissection on both sides of the septum. Once the deviated nasal The distance between the greater and lesser segments is propor-
septum is repositioned, nostril patency is reestablished on the tional to the width of the cleft lip. The segments will be approxi-
cleft side. mated from pressure exerted on them by the repaired cleft lip
The nasal floor is also reconstructed to eliminate the vestibu- molding the anterior element of the greater segment as shown
lar oral nasal communication if the palate is involved. A vomer in the preoperative (Figure 67-19) and postoperative (Figure
flap is used when possible to repair the anterior hard palate in 67-20) molds of cleft lip repairs.

Septoplasty repositions
the nasal septum

Restoration of patency
and natural width of
the nostril

FIG 67-18  Septoplasty with repositioning of the caudal aspect of the nasal septum.

Preoperative gap
between greater and
lesser segments

FIG 67-19  Gap between greater and lesser segments preoperative cleft lip repair.
958 CHAPTER 67  Techniques for Cleft Lip Repair

Postoperative narrowing of
gap between greater and
lesser segments

FIG 67-20  Reduction of gap between greater and lesser segments postoperative cleft lip repair.

A Incision
B
FIG 67-21  A and B, Raising a vomer flap into the anterior hard palate.

A Exposed bone
B
FIG 67-22  A and B, Insetting a vomer flap into the anterior hard palate.

Anterior Hard Palate THE SOUTH WALES CLEFT TEAM PROTOCOL FOR
A vomer flap is raised and inset into the cleft margin (Figure CLEFT LIP REPAIR: ADVANCEMENT/ROTATION LIP
67-21) to close the anterior hard palate at the time of repair of REPAIR WITH AN INFERIOR TRIANGULAR FLAP
the cleft lip to establish continuity of nasal floor closure (Figure
67-22). There may be some narrowing of the width of the pos- The authors close the cleft lip at 3 months old and the repair
terior cleft palate following vomer flap closure. involves the following key steps:
CHAPTER 67  Techniques for Cleft Lip Repair 959

1. An advancement/rotation repair of the cleft lip incorporat- side to the cleft side peak of the cupid’s bow curving parallel
ing an inferior triangular flap addressing the medial shorten- to and just above white line. It is curved medially and turns
ing and lateral displacement while restoring normal anatomy, across the vermilion through 90 degrees at the cupid’s bow
lip length, and symmetry peak and extends just past the wet-dry mucosal junction.
2. Achieving a functional muscular repair by restoring the 4. The incision on the lateral side extends from the alar base
displaced muscles to their correct anatomical position and along the junction of the nasal and lip skin to meet the
recreating the muscular rings of mid and lower face mucosa at 90 degrees (Figure 67-26). The incision then
3. Using a vomer flap to repair the anterior hard palate in extends from this point along the white roll parallel to the
continuity with the repair of the floor of nose skin/mucosal junction to the meet the upper point of the
4. Performing a McComb’s nasal dissection if required to lateral triangular flap.
release the lower lateral cartilage, allowing it to sit in a correct 5. An inferior triangular flap is incorporated along the incision
anatomical position in lateral element and inset into a backcut in the medial
5. Straightening of the caudal aspect of the nasal septum to element to lengthen the deficient medial element (Figure
place the septum in its correct central anatomical position 67-27). The triangle and the backcut must be the same
The key surgical steps in the authors’ surgical protocol are as height above the white roll. The addition of an inferior tri-
follows: angle to the advancement/rotation repair of a cleft lip allows
1. Right complete cleft lip: Identification of key surgical land- full restoration of length without the overextension of the
marks show on an illustration and clinical photograph medial incision under the base of columella. The lip length
(Figure 67-23). must not be shorter than normal side at end of surgery.
2. The skin markings include the following key landmarks 6. Advancement of the lateral side to align with the rotated
(Figure 67-24): short medial element (Figure 67-28).
• Cupid’s bow markings: Non-cleft side peak, trough and 7. The intranasal incisions are illustrated in Figure 67-29.
peak on the cleft side (a point on the cleft side equidistant The incision on the medial element extends at a tangent
from the trough of the cupid’s bow) to the curved incision, extending into the floor of the nose along
• First change in the appearance of white roll on the lateral the nasal/oral mucosal junction. It will connect to the vomer
element: The point of first change of the white roll and flap, and the raised flap will form the medial floor of the nose.
where the white roll and vermilion start to converge The incision on the lateral element is a continuation of the
• Base of columella: Remember it is rotated lateral element incision into the nose parallel to the junction of
• Alar base: The inferior lateral point the skin-mucosal junction. This incision extends to the alveolus
3. Curved incision along the short medial element (Figure and sits just below the inferior turbinate.
67-25): The aim is to place the scar in the line of the natural 1. Outline of sterile mucosa: The incisons are extended across
philtral ridge. The medial side of the philtrum must be the white roll medially and laterallly at 90 degrees to the
lengthened to match the non-cleft length. The incision does white roll. They extend though the wet–dry junction and
not extend along the base of columella at its superior aspect. then up into the nose. The mucosa outlined is the sterile
The incision extends from the base of columella on the cleft mucosa.

No extension across
base of columella
Incision extends across white roll
at 90 to cupid’s bow peak

Extends just past the wet/dry junction


A B
FIG 67-23  Left unilateral cleft lip. A, Diagram. B, Clinical
illustration. FIG 67-25  Curved incision on medial element.

Base of columella
Alar base
Peak of cupid’s bow on medial
side—equidistant from the trough First change in
compared with the non-cleft side the white roll on
lateral element
Peak and trough of
cupid’s bow

FIG 67-24  Skin markings on complete left unilateral cleft lip.


960 CHAPTER 67  Techniques for Cleft Lip Repair

Nasal skin
Short philtral length on Incision that separates nasal
the medial element skin from the lip skin
Normal philtral length on Lip skin
the non-cleft side Incision extending from the
junction of lip/nasal skin
with mucosa along the white
roll to the point of first change
in the white roll

FIG 67-26  Lateral skin incision.

Advancement of the lateral element


to upper landmark to base of columella
Inferior triangle placed just above the white mark on the medial segment
roll on the lateral side and inset into the
backcut on the medial side to lengthen the
skin deficiency on the medial side

Advancement of lateral element triangular


flap to the back cut on the medial segment
FIG 67-27  Inferior triangle and backcut. FIG 67-28  Advancement and rotation.

Tangential curve extending


into the floor of the nose along Continuation of the lateral
the nasal/oral mucosal junction element incision into the nose
parallel to the junction of the
skin-mucosal junction

FIG 67-29  Nasal incisions.

2. Advancement rotation skin markings with an inferior tri- 4. Subperiosteal dissection: This is performed if the transverse
angle (Figure 67-30): The vermilion border is tattooed with nasalis muscle will not reach the ANS. After the muscle
ink; these points act as landmarks to allow accurate recon- dissection is done, an incision is made in the buccal sulcus
struction of the white roll. Local anesthetic is infiltrated and up to the nasal margin of the cleft. A wide subperiosteal
marking incised. The sterile mucosa is excised, and the dissection is done from the vestibule on the cleft side over
muscle edges are identified along both sides of the cleft the piriform rim, nasal bone, and infraorbital and malar
margin. regions to lift the facial mask, taking care to protect the
3. Muscle dissection: The muscle is dissected on both sides of infraorbital nerve (Figure 67-33).
the cleft to separate the muscle from the mucosa and dermal 5. McComb nasal dissection26: This should be performed as
layers. On the medial side, dissection of the muscle is required if the lower lateral cartilage will not sit in the
carried out to relieve the abnormal attachments from ANS correct position when the nasal muscle is approximated.
and the columella allowing lengthening and rotation of the Access is gained to the lower lateral cartilages through a
skin deficiency on the medial element (Figure 67-31). On medial approach, allowing blunt dissection of the cartilage
the lateral side, muscle dissection is done to identify and from the overlying skin/nasal mucosa. The plain of dissec-
mobilize the head of the transverse nasalis and the length tion is subdermal and submucosal. The lower lateral carti-
of orbicularis (Figure 67-32). lage can also be approached from the lateral aspect if
CHAPTER 67  Techniques for Cleft Lip Repair 961

Sterile
Sterile
mucosa on
mucosa on
lateral side
medial side

FIG 67-30  Sterile mucosa on medial and lateral elements.

Muscle dissection from


ANS and deviation of base
of septum

FIG 67-31  Dissection of muscle on the medial side. ANS, Anterior nasal spine.

required, but this isn’t commonly performed (Figure from its attachment to the nasal spine and maxillary crest,
67-34). The McComb dissection reduces the buckling effect straightened and repositioned centrally (Figure 67-36).
of the lateral crus of the lower lateral cartilage, allowing it 7. Nasal floor reconstruction: The nasal floor is reconstructed
to sit in a more natural position. by suturing the hair-bearing nasal mucosa on both sides,
6. Septoplasty: Correction of the caudal aspect to the deviated posteriorly closing the lateral nasal floor to the vomer flap
nasal septum provides stability and exact positioning of the (Figure 67-37).
previously lifted alar crus of the cleft side and nasal tip. This 8. Suturing of transverse nasalis to ANS/septum: This is
allows the nose to grow in a balanced way with equal muscu- done to secure the position of the base of the septum
lar force being exerted on both sides. The caudal aspect of the (Figure 67-38).
nasal septum is then carefully isolated and freed through the 9. Natural approximation of orbicularis oris: The orbicularis
same cleft incision by splitting and raising the perichon- is approximated and sutured to a natural position main-
drium on both sides (Figure 67-35). The septum is detached taining the muscle length (Figure 67-39).
962 CHAPTER 67  Techniques for Cleft Lip Repair

Muscle dissection
identifying head of
transverse nasalis

FIG 67-32  Dissection of muscle on the lateral side.

Infraorbital nerve foramen

Subperiosteal dissection
extending from piriform rim
to malar prominence

FIG 67-33  Subperiosteal dissection on lateral side.

Nasal cartilage

Medial blunt
dissection

FIG 67-34  McComb nasal dissection.


CHAPTER 67  Techniques for Cleft Lip Repair 963

Exposure of the
caudal aspect of
the deviated nasal
septum

FIG 67-35  Exposure of the deviated caudal aspect of the nasal septum.

Detachment of septum from


ANS and straightening of
septum

FIG 67-36  Detachment and straightening of caudal aspect of nasal septum. ANS, Anterior nasal
spine.
964 CHAPTER 67  Techniques for Cleft Lip Repair

Reconstruction of
floor of nose

FIG 67-37  Reconstruction of nasal floor.

Suturing of
transverse
nasalis to
ANS/septum

FIG 67-38  Suturing of transverse nasalis to anterior nasal spine (ANS)/septum.

10. Skin closure: Tension-free skin closure is performed (Figure lateral side.27 The incision is then bowed in a curvilinear fashion
67-40). This is made possible by the subperiosteal dissec- near the base of the columella crossing the philtrum toward the
tion, radical mobilization, and suturing of the transverse far lateral extent of the columellar base (Figure 67-41).28 In one
nasalis and orbicularis muscles. form or another, it is the most widely practiced method today.
The rotation advancement technique relies on a “cut as you
COMMON TECHNIQUES OF CLEFT LIP REPAIR go” strategy that allows continuous modifications during the
design and execution of the repair. It does not adhere to strict
Millard Cleft Lip Repair geometrical principles or measurements.
Flap Design However, it may leave a scar crossing the midline at the base
The Millard cleft lip repair is based on a rotation flap on the of the columella and cause shortening of the lip on the cleft side
medial cleft side coupled with an advancement flap on the cleft with resultant vermilion notching and whistle deformity.
CHAPTER 67  Techniques for Cleft Lip Repair 965

Natural
approximation of
orbicularis oris

FIG 67-39  Functional repair of orbicularis oris.

FIG 67-40  Tension free closure of skin.

Delaire Cleft Lip Repair Pfeiffer Cleft Lip Repair


The Delaire cleft lip technique incorporates a curvilinear inci- Flap Design
sion that extends up and parallels the medial cleft margin but Pfeifer described a wavy-line repair that allowed downward
stops near the medial edge of the base of the columella.29 The rotation as the curves were approximated into a straight line.31
lateral lip element advancement incision curves outward and The two curves are brought together such that the highest and
then medially as it extends superiorly before it again extends to lowest points of one curve are approximated with the corre-
the alar base (Figure 67-42).30 sponding highest and lowest points of the other, thus creating
Delaire does not incorporate an inferior triangle into a straight line (Figure 67-43).
his cleft lip repair, which limits the extra length that can The Pfeiffer repair achieves good vertical length soft tissue
be gained. A Delaire cleft lip repair does not include a reconstruction; but in some cases, this can be at the expense of
vomer flap. soft tissue reconstruction in the horizontal dimension.
966 CHAPTER 67  Techniques for Cleft Lip Repair

FIG 67-41  Millard repair. FIG 67-44  Afroze repair.

FIG 67-42  Delaire repair. FIG 67-45  Tennison repair.

Tennison Cleft Lip Repair


Flap Design
The triangular flap repair was initially described in 1952 by
Tennison.33 Tennison’s technique made use of an inferior
backcut that begins not far above the cleft-side peak of the
cupid’s bow of the medial lip element and angled superolater-
ally. Precisely measured triangle flaps allow vertical lengthening
of the medial lip element while enabling lengthening of what is
often an otherwise short transverse lateral lip element without
compromising the ideal basal position of the philtral column
incision (Figure 67-45).34
The main disadvantage of the triangular flap repair tech-
FIG 67-43  Pfeiffer repair. nique is that the philtrum on the cleft side is violated by the
triangular flap. Some authors believe this leaves a more notice-
able scar. Another potential disadvantage is the difficulty in
modifying the repair or performing secondary revision at a later
Afroze Cleft Lip Repair stage due to the zigzag scars.
Flap Design
The Afroze incision is a combination of two incisions—the Fisher Cleft Lip Repair
Millard incision on the medial cleft side and the Pfeiffer incision Flap Design
on the cleft side (Figure 67-44).32 The flap is designed so that The repair allows for a repair line that ascends the lip at the
the Millard flap on the cleft side rotates downward, and the peak seams of anatomical subunits. Applying the principle of ana-
of the distal curve on the Pfeiffer flap is positioned in the tri- tomic subunits to cleft lip repair, the “ideal line of repair” should
angular defect formed by the downward movement of the be one that ascends the lip from the cleft-side peak of cupid’s
Millard flap. bow to the base of the nose along a line exactly mirroring
The lip scar does not lie along the philtrum; this is the only the non–cleft-side philtral column (Figure 67-46).35,36 These
disadvantage of this technique. However, the scar heals excep- maneuvers help preserve natural subunit boundaries, allowing
tionally well because the wound is closed under no tension for rotation and medial lip lengthening at the expense of nar-
whatsoever. rowing the philtrum to a degree.
CHAPTER 67  Techniques for Cleft Lip Repair 967

Fisher often adds a small inferior triangle just above the and use of geometric and curvilinear incisions to approach a
cutaneous roll for additional rotation and feels that this accen- vertically oriented closure.
tuates the pout of the lip. Although this technique is included
in the category of rotation/advancement repairs, it is clearly a
hybrid of multiple principles, including triangle-flap techniques REPAIR OF THE INCOMPLETE CLEFT LIP
The repair of the incomplete cleft lip follows the same principles
as outlined for the repair of the complete cleft lip. The incision
is modified to divide the nasal skin from the lip skin in a line
along the base of the nasal sill (Figure 67-47). The texture of
the nasal skin is different from the texture of the skin of the lip,
and the incision line is placed between the junction of both skin
types. The texture of the skin that is excised is abnormal due to
the lack of underlying muscle insertion. A small wedge excision
into the nasal sill may be used to prevent a prominence of scar
tissue from developing in this area. The authors do not include
this extension into the nasal sill, because it may lead to nostril
narrowing and flattening of the nasal sill. If a tissue prominence
should develop, it can be easily treated with a wedge excision at
FIG 67-46  Fisher repair. a later date.

Skin of the floor


of the nose

Lip skin

Sterile mucosa
to be excised

C
B

FIG 67-47  A, An incomplete left side cleft lip. B and C, Preoperative skin markings for an incom-
plete left side cleft lip repair.
968 CHAPTER 67  Techniques for Cleft Lip Repair

FIG 67-48  Postoperative result for the repair of an incomplete


left side cleft lip. FIG 67-49  Asymmetric bilateral cleft lip and palate—incomplete
on the right side and complete on the left side.

The postoperative result for an incomplete cleft lip repair is


illustrated in Figure 67-48. cleft lip, this small caseload could be considered suboptimal for
a condition that presents the following unique set of technical
SURGICAL REPAIR OF THE BILATERAL CLEFT LIP problems:
• Prolabial mucosa: The isolated prolabium, which is deprived
The incidence of bilateral cleft lip (Figure 67-49) and palate is of muscle and normal vermilion, protrudes anteriorly and is
much lower than that of unilateral cleft lip and palate, and it rotated upward. Its dimensions are reduced by secondary
makes up less than 10% of all cleft deformities. Of all the dif- hypoplasia and the frequent absence of lateral incisors. Some
ferent types of cleft deformity, the complete bilateral cleft lip surgeons advocate partial retention of the prolabial mucosa
and palate presents the greatest challenge to the cleft team. to recreate a cupid’s bow. The authors do not retain the
There is very little conformity in the technique and timing of prolabial mucosa, because its long-term appearance can be
repair of bilateral cleft lip in the United Kingdom. Repair of a dry and abnormal if retained. The authors prefer to recreate
bilateral cleft lip is characterized by a huge diversity of treat- the central white roll as a scar line between prolabial skin
ment protocols that are often associated with more long-term and mucosa.
issues than unilateral clefts. • Nasal deformity: The bilateral nasal cleft is seldom corrected
The key principles of repair of the bilateral cleft lip that are by functional surgery alone, and the appearance of a repaired
followed are the same as for the unilateral cleft lip. Advance- bilateral cleft lip is often dominated by the short, tethered
ment of the displaced lateral element and the need to regain appearance of the columella and broad nasal tip. An under-
length in the short medial element are the same in bilateral as standing of the true nature of the nasal deformity in bilateral
in unilateral cleft lips (Figures 67-50 and 67-51). cleft lip has gradually emerged with the simple observation
The prolabial skin is raised exposing to allow functional that the “columella is in the nose.”37-39 Some surgeons
reconstruction of the muscles across the premaxilla perform primary repair on the nose to retrieve the columella
(Figure 67-52). from the nose and restore the prominence of the nasal tip at
Muscle reconstruction is the same as in unilateral cleft lips, an early age. However, the authors’ preferred protocol is to
except the two halves of orbicularis and transverse nasalis avoid primary nasal surgery, because it produces scarring
muscles are sutured together under the prolabium and trans- and fibrosis that may result in unfavorable growth and makes
verse nasalis fixed to the ANS (Figures 67-53 and 67-54). a secondary rhinoplasty procedure more difficult. Instead,
The final closure of the bilateral cleft lip is illustrated in the authors perform nasal surgery at a preschool age if the
Figure 67-55. The functional repair of the muscle across the nasal deformity is significant or more ideally in the late teens
prolabium retracts the protruding premaxilla back into align- at the end of growth. The timing of this is decided by the
ment with the lesser segments provided that there hasn’t been parents’ wishes.
transverse collapse of the lesser segments. The tip of the nose is • Asynchronous repair: In very severe forms of bilateral cleft
flat and depressed, but this is addressed at secondary rhino- lip, the cleft may be repaired one side at a time (i.e., a one
plasty, which is ideally performed when the patient has finished side first approach). Alternatively, a lip adhesion may be
growing (i.e., in their late teens). performed, whereas repair of the muscles is usually com-
Although the surgical principles of repair of bilateral cleft lip pleted at the second stage. Bilateral lip adhesion helps to
are similar to those applied to the management of unilateral reposition the protrusive premaxilla, which enables a later
CHAPTER 67  Techniques for Cleft Lip Repair 969

Rotation of the medial elements

Nasal skin

Advancement of the
Lip skin lateral elements

Sterile mucosa for excision


along the prolabium
FIG 67-50  Skin markings for repair of a bilateral cleft lip.

FIG 67-52  Excision of sterile mucosa to expose muscles.


FIG 67-51  Skin markings for repair of an asymmetric bilateral
cleft lip.

Suture skin of
floor of nose

Suture through
transverse nasalis

FIG 67-53  Functional reconstruction of transverse nasalis to anterior nasal spine.


970 CHAPTER 67  Techniques for Cleft Lip Repair

Reconstruction of both
halves of the orbicularis oris
across the premaxilla

FIG 67-54  Functional reconstruction of orbicularis oris muscle repair under the prolabium.

FIG 67-55  Repaired bilateral cleft lip.

definitive repair. This inevitably delays any attempt at syn- is technically demanding for both the orthodontist and the
chronous repair of the lip and nose, and there is no evidence patient’s carers, and it would benefit from being submitted
that it improves long-term outcome. The authors’ preferred to a randomized controlled trial before being universally
method of repair of bilateral cleft lip is a synchronous repair adopted as a useful adjunct to the already considerable
at 3 to 4 months of age, but they recognize that occasionally burden of care suffered by patients with bilateral clefts. The
in very wide clefts it may not be technically possible to repair use of NAM as an adjunct to primary repair of the lip and
the muscles completely. nose has been a recent development that may help improve
• Pre-surgical orthopedics: Formal surgical repair is often nasal outcome.
preceded by pre-surgical orthopedics to retroposition the
premaxilla and enable a tension-free repair of the lip.40 Pre-
surgical orthopedics may be of benefit in the surgical repair PITFALLS
of bilateral cleft lip by helping achieve complete reconstruc- • Highly specialized surgery is necessary in the early months of life to
tion of the muscles across a severe cleft with a protuberant maximize function of the face and oro-pharynx. Deficiencies of facial
premaxilla and by lengthening the columella. Although there and dental development, speech and hearing remain frustratingly
common and may be accompanied by psycho-social issues.
is no doubt that it facilitates the repair and is still widely
• Evidence for and against the use of the vomer flap is conflicting its
used, there is no evidence that it improves outcome.41 use serves to narrow the hard palate as it heals by secondary inten-
• Nasoalveolar molding (NAM): This is a form of pre-surgical tion. However, avoiding its use would cause less scarring and leave
orthopedics that expands the columella and repositions the the alveolus in pristine condition for bone grafting. Ultimately this
premaxilla. Columellar lengthening allows the cartilages of might lead to better facial growth.
the nasal tip to be reshaped.42-44 Early studies have shown • Repairing the soft palate at the time of lip repair also encourages the
cleft in the hard palate to narrow due to muscular action. However,
that NAM does this effectively before operation. Although
this may increase the risk of a fistula developing at the hard/soft
early results with preoperative NAM are promising, objective palate junction.
analysis of long-term outcome has not yet been reported. It
CHAPTER 67  Techniques for Cleft Lip Repair 971

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